Home Journals Articles Updates About
CN
image
收藏切换
USB1 deficiency disrupts neutrophil maturation via RNA dysregulation independent of global pre-mRNA splicing
收藏切换
PDF
Hang Li, Guiying Shi, Jiaming Tang, Yiying Huang, Jie Wang, Xuepei Lei, Lin Bai
Animal Models and Experimental Medicine | 2026, 9(7) : 1353 - 1363
Less
收藏切换
Animal Models and Experimental Medicine | 2026, 9(7): 1353-1363
• Regular Articles •
USB1 deficiency disrupts neutrophil maturation via RNA dysregulation independent of global pre-mRNA splicing
Full
Hang Li, Guiying Shi, Jiaming Tang, Yiying Huang, Jie Wang, Xuepei Lei, Lin Bai
Affiliations
  • State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS, Ministry of Education, Beijing, China
Published: 2026-07-28 doi: 10.1002/ame2.70206
Outline
收藏切换
Background:

U6 biogenesis 1 (USB1) gene mutations cause poikiloderma with neutropenia (PN), which is clinically characterized by skin hyperpigmentation, nail dysplasia, neutropenia, and an elevated risk of cancer. USB1 functions as an RNA exonuclease involved in RNA maturation and stability regulation, although its precise mechanism of action in the hematopoietic system remains unclear.

Methods:

We established a myeloid cell-specific USB1 knockout mouse model (USB1fl/fl-Lyz2-cre) using CRISPR/Cas9. Using a combination of research methods, including Western blot, flow cytometry, messenger RNA (mRNA) sequencing, micro-RNA (miRNA) sequencing, and quantitative polymerase chain reaction (qPCR), we investigated the effects of USB1 deficiency on neutrophil development and differentiation, along with the underlying signaling molecular mechanisms.

Results:

Experimental results indicated that USB1 deficiency in mouse myeloid cells not only leads to dysregulation of miRNA expression but also interferes with the developmental, differentiation, and maturation processes of neutrophils by affecting key genes, such as IL1a, Selp, and Kilt. This impact can be traced back to the stages of myeloid progenitor cells.

Conclusions:

USB1 influences neutrophil maturation and myeloid progenitor cell differentiation by regulating the expression of specific miRNAs and mRNAs. This provides novel in vivo experimental evidence for understanding the pathogenesis of PN in patients.

microRNA  /  myeloid knockout mice  /  neutrophil maturation  /  USB1
Hang Li, Guiying Shi, Jiaming Tang, Yiying Huang, Jie Wang, Xuepei Lei, Lin Bai. USB1 deficiency disrupts neutrophil maturation via RNA dysregulation independent of global pre-mRNA splicing[J]. Animal Models and Experimental Medicine, 2026 , 9 (7) : 1353 -1363 . DOI: 10.1002/ame2.70206
Full Text
Less
Funding
Less
  • National Key Research and Development Project(2022YFA1103803)
  • CAMS Innovation Fund for Medical Sciences(2021-I2M-1-035)
Appendix
Less
Year 2026 volume 9 Issue 7
PDF
53
14
Cite this Article
BibTeX
Article Info
doi: 10.1002/ame2.70206
  • Receive Date：2026-01-09
  • Online Date：2026-08-06
  • Published：2026-07-28
Article Data
Affiliations
History
  • Received：2026-01-09
  • Revised：2026-03-12
  • Accepted：2026-03-16
Funding
National Key Research and Development Project(2022YFA1103803)
CAMS Innovation Fund for Medical Sciences(2021-I2M-1-035)
Affiliations
    State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS, Ministry of Education, Beijing, China

Corresponding:

Lin Bai, State Key Laboratory of Respiratory Health and Multimorbidity, Key Laboratory of Pathogen Infection Prevention and Control, Institute of Laboratory Animal Science, National Human Diseases Animal Model Resource Center, National Center of Technology Innovation for Animal Model, Peking Union Medical College, CAMS, Ministry of Education, Beijing, 100021, China. Email:
References
Share
https://castjournals.cast.org.cn/joweb/amem/EN/10.1002/ame2.70206
Share to
QR

Scan QR to access full text

Cite this article
BibTeX
Citations
表12种不同金属材料的力学参数
科
Family		 属数
Number of
genus		 种数
Number of
species		 占总种数比例
Percentage of
total species (%)		 属
Genus		 种数
Number of
species		 占总种数比例
Percentage of total
species (%)
鹅膏菌科Amanitaceae 2 11 5.26 鹅膏菌属 Amanita 10 4.78
小菇科 Mycenaceae 2 12 5.74 丝盖伞属 Inocybe 5 2.39
多孔菌科 Polyporaceae 8 14 6.70 蜡蘑属 Laccaria 5 2.39
红菇科 Russulaceae 3 23 11.00 小皮伞属 Marasmius 6 2.87
小菇属 Mycena 11 5.26
光柄菇属 Pluteus 5 2.39
红菇属 Russula 17 8.13
栓菌属 Trametes 5 2.39
关闭全屏
  • BibTeX
  • EndNote
  • RefWorks
  • TxT
Links
Articles
Latest Articles
Most Read
Collections
Updates
Events
News
Multimedia
About
About Us
Contact
No. 86 Xueyuan South Road, Haidian District, Beijing
100081
010-62199257
qkjq@cast.org.cn

Copyright © 2025 China Association for Science and Technology. All rights reserved. For all open access content, the relevant licensing terms apply.
Sponsored by the Office of the Leading Group for Cybersecurity and Informatization of CAST, and supported by Science and Technology Review Publishing House